An unusual case of a desmoplastic fibroma of the temporal bone is presented. Although classified benign, this intraosseous lesion exhibits local aggressiveness and has a high potential for recurrence. This rare condition occurs predominantly in the mandible and in the long bones and is seldom seen in the calvarium. Here we add another case to the previously described eight in the skull and this is the fourth such case reported in the temporal bone. The clinical features, radiology, histopathology and the therapeutic considerations of this lesion in a 72-year-old female are discussed. In addition, a literature review of all the cases affecting the skull bones is presented.